1. CMR of Non-ischemic Dilated and Restrictive Cardiomyopathies
Frederick L. Ruberg, MD
Director, Advanced Cardiac Imaging Program
Section of Cardiology, Department of Medicine
Department of Radiology
Boston University School of Medicine
Boston Medical Center
March 2, 2009

2. Utility of CMR in LV systolic dysfunction
Diagnosis
Ischemic vs. Non-ischemic
Etiology
Prognosis
Functional recovery with treatment
Morbidity and mortality

3. Case Presentation
58 year old woman with class II-III HF symptoms referred for echo

4. Case Presentation

5. Why obtain CMR next?
Precise quantification of LV and RV function and volumes from cine images
Permit detection of improvement or decrement with treatment
Quantification of associated valvular regurgitation
Visualization of fibrosis or infarction (DE/LGE)
Pattern of DE important to differentiate etiology
Afford predictors of recovery
Afford predictors of CRT efficacy

6. LGE Imaging: Initially for scar
Kim RJ et al., Circulation 1999

7. Fibrosis Imaging by DE/LGE
Imaging min after gadolinium (0.1 to 0.2 mmol/kg)
Retained contrast in regions of fibrosis or infarction
No contrast in normal myocardium
Marholdt EHJ 2005

8. Ischemic DE Pattern by CMR
Marholdt EHJ 2005

9. Differentiation of Ischemic vs. Non-ischemic CMP
90 patients with CHF and LV dysfunction obtained cardiac cath and CMR
70% without CAD by cath
59% no DE
28% mid-wall DE
13% sub-endocardial DE (mis-assigned)
30% with CAD and history of MI
100% with sub-endocardial DE
McCrohon et al. Circ 2003

10. Ischemic vs. non-ischemic
McCrohon et al. Circ 2003

11. Case Example – Ischemic or Non-
35 year old male with severe LV dysfunction
TSH > 120

12. Case Example – DE images

13. Mid-wall enhancement
Not subendocardial, does not follow infarction pattern
Most frequently septal
Lower signal intensity vs. MI
Etiology and significance is controversial

14. Mid-wall enhancement: Morbidity and Mortality
101 patients with dilated CMR underwent CMR and were followed for 685 days
35% had mid-wall enhancement
Increased risk of death or hospitalization (OR 3.4)
No difference in mortality
Increased likelihood of SCD/VT (OR 5.2)
Persisted after correcting for LVEF
Assomoul et al. JACC 2006

15. Mid-wall enhancement: Morbidity and Mortality
Assomoul et al. JACC 2006

16. Histologic correlate of mid-wall
Assomoul et al. JACC 2006

17. Mid-wall enhancement: Morbidity and Mortality
A. Mortality or hospitalization
for CV cause
B. Adjusted for age, LV/RV EF, LV volumes, digoxin VT
VT Adjusted for LVEF
Assomoul et al. JACC 2006

18. DE confers increased risk
65 patients with non-ischemic dilated CMP, EF < 35%, underwent CMR at baseline, followed for 17 months
42% showed LGE at baseline
Non-ischemic pattern
44% of those with LGE had adverse event vs. 8% without (HF, ICD discharge, death)
Wu, JACC 2008

19. DE and risk in non-ischemic CMP
Wu, JACC 2008

20. Functional Recovery with Medical Treatment
45 patients with CHF treated with beta-blocker, CMR with DE at baseline and 6 month follow-up
62% ischemic (of those 100% with DE)
38% non-ischemic (of those only 2% with DE)
Transmurality of DE predicted contractile improvement, change in EDV and ESV
Bello et al. Circ 2003

21. Functional Recovery with Medical Treatment
Bello et al. Circ 2003

22. Prediction of CRT outcome by CMR
23 patients who qualified for CRT underwent CMR at baseline, follow-up at 3 months for wall motion, 6 min walk, QOL
50% history of MI
57% demonstrated response
DE amount lower in responders
<15% of LV mass – 85% sens., 90% spec.
Septal transmurality of < 40% - 100% sens/spec.
White et al. JACC 2006

23. Prediction of CRT outcome by CMR
White et al. JACC 2006

24. Conclusions for dilated CMR
Absence of any DE is good (non-ischemic)
Predicts likelihood of recovery
Better outcomes with CRT
Lower likelihood of events

25. Case Example – cine CMR

26. Case Example – DE CMR

27. Case Example Symptomatic improvement with ARB, beta blocker
Referred for CRT

28. Case Example
58 year old woman with class II-III HF symptoms referred for echo

29. Case Example
HF with preserved LV function, grade II-III diastolic dysfunction

30. Differential Diagnosis
Etiology in this case is more important
Hypertensive remodeling
Hypertrophic Cardiomyopathy
Infiltrative Cardiomyopathy
Amyloidosis
Storage disease (Anderson Fabry)
Heavy metal deposition (hemochromatosis)

31. Utility of CMR
Not necessary to define LV volumes, although mass quantification useful
DE CMR
Etiology
Prognosis

32. Does LVH from HTN have DE?
83 patients with LVH from AS (25%), HTN (31%), and HCM (44%) underwent CMR
DE seen in all etiologies
AS 62%, HTN 50%, HCM 72%
Only distinctive pattern from HCM
Generally associated with increased mass
Rudolph, JACC 2009

33. CMR in LVH
Rudolph, JACC 2009

34. LVH with CHF

35. CMR in Amyloidosis Abnormally long myocardial T1 after Gd
Normal ≈ 1100 ms, amyloid ≈ 1400 ms
Rapid clearance of gadolinium from blood pool, abnormal distribution kinetics
Render blood pool dark
Challenging to obtain optimal myocardial nulling
Global, sub-endocardial pattern described
Maceira et al. Circ 2005, Krombach, JMRI 2007

36. CMR in Amyloidosis
Maceira: Circulation 2005

37. CMR in Amyloidosis Normal protocol Modified amyloid protocol
0.1 to 0.2 mmol/kg wait mins
Modified amyloid protocol
0.1 mmol/kg wait 5 mins
Diffuse DE, poor myocardial nulling

38. Diffuse DE seen in Cardiac Amyloidosis
Van den Driesen et al. AJR 2006

39. Performance of CMR in Amyloid
Sensitivity 80%, specificity 94%, PPV 92%, NPV 85%
Vogelsberg et al, JACC 2008

40. CMR predictors of events
Amount or presence of DE does not predict mortality
Amount of DE relative to LV mass does correspond to heart failure symptoms
Ruberg et al, AJC 2009

41. CMR in Cardiac Amyloidosis
Amyloidosis with cardiac involvement
Amyloidosis without cardiac involvement
Ruberg et al,AJC 2009

42. CMR in Cardiac Amyloidosis
Ruberg et al,AJC 2009

43. CMR in Cardiac Amyloidosis
Intramyocardial T1 gradient between epi- and endo-cardium predictive of survival
DE/LGE was not
Maceira et al, JCMR 2009

44. CMR in Anderson Fabry
32 Fabry patients treated with a-glactosidase, CMR obtained at baseline, followed for 3 years
63% had fibrosis by DE, 27% did not
Absence of fibrosis associated with improved function, reduced mass, improved exercise capacity
Weidemann et al., Circ 2009

45. CMR in Anderson Fabry
Weidemann et al., Circ 2009

46. CMR in hemochromatosis
T2* weighted imaging
T2* abnormally shortened in iron deposition
Widely explored for thalassemia
With chelation treatment (deferoxamine/deferiprone), T2* increases correlate to functional improvement in LVEF
Tanner et al. Circ 2007

47. Case Example – DE Images

48. Case example Diagnosis: Amyloidosis
LGE present but can tell patient not predictive of poor outcomes
Underwent stem cell transplant in 2005, doing well today, HF symptoms are controlled

49. Conclusions In dilated CMP, absence of DE portends:
Recovery of LV function with medical treatment
Lower likelihood of death or hospitalization for HF
Higher likelihood of response to CRT
In dilated CMP, presence of DE
Identification of ischemic etiology and provides information in respect to revascularization recovery
Increased risk of adverse event and lower CRT response

50. Conclusions In CMP with LVH/wall thickening, CMR with DE imaging can:
Identify etiology of CMP
Follow response to treatment
Associate with clinical outcomes
CMR with DE is useful as baseline exam in all forms of cardiomyopathy